Kosmidis et al. Journal of Orthopaedic Surgery and Research 2010, 5:56 pptx

CAS E REP O R T Open Access
Hemolymphangioma of the lower extremities in
children: two case reports
Ilias Kosmidis
1*
, Maria Vlachou
2
, Anastasios Koutroufinis
1
, Konstantinos Filiopoulos
1
Abstract
Background and purpose: Hemo-lymphangiomas are rare benign tumors that arise from congenital malformation
of the vascular system. They are usually diagnosed at birth or early in childhood. The management of hemo-
lymphangiomas in children remains challenging because complete resection is often difficult to be achieved and
recurrences are common.
Methods: We present the case of two children with a mass on their left tibia. Imaging modalities, plain radiograph,
Ultrasonography and Magnetic Resonance were used to investigate the nature of the mass, the anatomical
relationship to the neighboring tissues and help planning the surgical resection. The dominant diagnosis was
hemo-lymphangioma. Both lesions increased in size in a short period of follow-up thus we decided to proceed to
surgical excision.
The diagnosis of hemo-lymphangioma was confirmed by histological examination of the surgical specimen.
Post-operatively, seroma was formed to the first patient, managed by placing a drainage and immobilizing the
limb on a splint.
The second patient experienced no complications post-operatively.
After 12 months of follow-up both patients had no complications or recurrence.
Conclusions: Very few cases of hemo-lymphangiomas of the extremities have been reported in the literature.
Those tumors can grow slowly and remain asymptomatic for a long period of time or may become aggressive and
enlarge rapidly, without invasive ability though.
Radical resection is the choice of treatment offering the lowest recurrence rates. Other therapeutic methods are:
aspiration and drainage, cryotherapy, injection of sclerotic agents and radiotherapy; although none of those offers

better results that the surgical excision.
Introduction
Lymphangiomas are a heterogeneous group of vascular
malformations of the lymphatic channels composed of
cystically dilated lymphatics. According to Landing and
Farber [1], those benign malformations, are classified in
four categories: capillary lymphangioma, cavernous lym-
phangioma, cystic lymphangioma (hygroma) and hemo-
lymphangioma (combination of hemangioma and
lymphangioma).
The latter, congenital malformation, can remain
asymptomatic for a long period of time. On the other
hand, it may grow rapidly, surrounding or infiltrating
the neighboring tissues or ot her major structures, thus
making the excision a real challenge for the physician
[2,3].
Hemo-lymphangiomas are in most c ases detected at
birth or early in a child’s life, usually before the age of
twoyears.Alternatively,withtheintroductionofthe
prenatal ultrasound, the diagnosis can be placed in the
uterus [4].
This essay is a case report of two children with hemo-
lymphangiomas of the lower extremities and its purpose
is to define the most effective therapeutic approach of
those lesions. Written parental permission was obtained
to allow t he use of confidential information held in the
hospital’s records, as Institutional Review Board (IRB)
does not exist in our country.
* Correspondence:
1

Orthopaedic Clinic, Penteli’s Children Hospital, Athens, Greece
Full list of author information is available at the end of the article
Kosmidis et al. Journal of Orthopaedic Surgery and Research 2010, 5:56
/>© 2010 Kosmidis et al; licensee BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative
Commons Attribution License ( y/2.0 ), which permits unrestricted use, dist ribution, and
reproduction in any medium, provide d the origi nal work is properly cited.
Case 1
A two month old female infant presented to the ortho-
pedic examination room with a palpated mass on the
anteromedial side of the proxim al left tibia. On the
physical examination the lesion was found to be oval
in shape, soft, compressible and painless. Anteropos-
terior and lateral plain radiographs demonstrated swel-
ling of the soft tissue at this part of the tibia without
signs of bony erosion (Fig. 1). The ultrasound (Doppler
sonography) detected a cystic lesion with blood flow,
measured 12 mm × 4 mm, while the integrity of the
bone cortex was confirmed. The most possible diagno-
sis was hemo-lymphangioma. In the following six
months, despite the fact that the size of the mass
increased, the patient remained asymptomatic. A new
ultrasound was performed (Fig. 2) and measured the
mass 23 mm × 6 mm; the origin remained cystic. In
the yearly follow-up the lesion’s s ize increased to 44
mm × 37 mm.
Due to the continuous augmentation of the mass, sur-
gical excision was decided. An MRI (Fig. 3) was per-
formed, pre-operatively, in order to establish the extent
ofthetumoranddefinetherelationshiptothesur-
rounding structures. A well-defined extra-articular cystic

malformation with a maximum diameter of 45 mm was
viewed, located on the anteromedial s ide of the left
tibia. The lesion had fine adhesions to the surrounding
tissues and the cortex of the bone was found intact.
En bloc resection was performed releasing the speci-
men from the adhesions to the subcutaneous tissues
and the medial head of the gastrocnemious. Macroscopi-
cally, it was found oval in shape with harsh features and
spotty surface (Fig. 4).
Histological examination described the resec ted tumor
as a fibro-lipomatous mass containing dense fibrous
conjunctive tis sue with vascular areas of lymphatic cells
and vessels filled in with red blood cells (Fig. 5 & 6).
The definitive histological diagnosis was hemo-
lymphangioma.
Ten days post-operatively the trauma area was swel-
ling; the seroma formed was aspirated and gave 20 ml
of sterile, bloody fluid.
Figure 1 Case 1- Plain radiograph of the left tibia,
demonstrating the mass in the inner-upper part.
Figure 2 Case 1- In a s ix month f ollow-up, pre-operative
Ultrasound measures the tumor 23 mm × 6 mm.
Kosmidis et al. Journal of Orthopaedic Surgery and Research 2010, 5:56
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A week later, the swelling recurred; surgical explora-
tion and lavage was performed, while a drainage was
placed. Intravenous Netilmicin and Cefamandile Nafate
were administered for a total period of ten days,
whereas the leg was immobilized on an above knee
splint. The swelling was progressively decreased. A week

after, the trauma area was examined by the ultrasound
and 1,58 m l’s of fluid were demonstrated. A full plaster
was placed with the knee joint flexed in 90° for three
weeks. At the end of that period of time the volume of
the fluid was less that 1 ml. After two months of follow-
up by clinical and Ultrasonography examination, the
quantity minimized to zero, while in the yearly follow-
up no recurrence was observed.
Figure 3 Case 1- MRI defines the margins of the tumor.
Figure 4 Case 1- Perioperative aspect of the mass.
Figure 5 Histological examination of the mass . Collapsed and
infolded cyst wall.
Figure 6 Histological examination.Cystwallstructureshowing
loose connective tissue stroma lined by flat endothelial cells,
beneath which is obvious a lymphocytic infiltration.
Kosmidis et al. Journal of Orthopaedic Surgery and Research 2010, 5:56
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Case 2
The secon d case regards a 5 year-old male patient , with
a palpable, painless mass on the anterior side of the
proximal left tibia, located 2 cm below the tibial tuber-
cle. The tumor, w ithin a two month follow-up, was
doubled in size; although it remained asymptomatic. A
soft tissue swelling and intact bone cortex were demon-
strated by the plain radiographs (anteroposterior and
lateral views). The ultra sound performed depicted a cys-
tic mass with moderate vascularization, measured 21
mm × 18 mm × 5 mm (Fig. 7).
The treatment decided was en bloc resection of the
tumor, followed by rigorous cauterization of the blood

and lymphatic vessels; a drainage was placed.
Microscopically, the tumor examined, was described
as a soft tissue cystic mass consisted of lymphatic and
blood vessels; the stroma was infiltrated by lymphocytes.
The histological diagnosis was hemo-lymphangioma.
Cephalosporin was administered, in 3 doses totally,
pre and post-operatively, while the limb w as immobi-
lized on a splint with the knee joint flexed in 30° for a
total period of three weeks. In the trauma area no ser-
oma was formed thereby, we removed the drainage.
At the yearly follow-up no recurrence was observed,
verified by the use of Ultrasonography (Fig. 8).
Discussion
Hemo-lymphangiomas are rare benign tumors that
appear to arise from congenital malformation of the vas-
cular system. The formation of that tumor may b e
explained by obstruction of the venolymphatic commu-
nication, between dysembrioplastic vascular tissue and
the systemic circulation [5]. Hemo-lymphangiomas are
mostly presented as cystic or cavernous lesions.
Very few cases of hemo-lymphangiomas of the extre-
mities have been reported in the literature. A retrospec-
tive study of one hundred and eighty-six (186) patients’
presenting one hundred and ninety-one (191) hemangio-
mas was published; their anatomical location was: 48%
in the head and neck, 42% in the extremities and 10% in
internal or visceral locations. Histological examination
revealed that only three of them had combined hemo-
lymphangiomas [6]. Macroscopically, complete excision
gave the best results with lower recurrence rate. On the

contrary, aspiration and injection of sclerotic agents
gave the highest risk of recurrence. Among the most
important risk factors for recurrence are: anatomical
location of the lesion, size, complexity and surgical tech-
nique. In that study, the average follow-up period was
three years, while 95% of the patients completed a suffi-
cient period of twelve months.
The incidence of hemo-lymphangiomas varies from
1,2 to 2,8 per 1000 newborns[7] however, only 6,5% of
them are loc ated on the extremities [8,9]; both sexes get
equally affected. The diagnosis in most cases (90%) is
placed before the age of two yea rs [2], while 60% of
those are present at the time of birth.
The clinical on set of hemo-lymphangiomas can vary
from a slowly growing cyst over a period of years to an
aggressive enlarging tumor, without invasive ability.
Their size varies due to the anatomical location and
relationship to the neighboring tissues. Small tumors are
usually supe rficial, while the large ones are located dee-
per and have cystic texture. The most common compli-
cations are random or traumatic hemorrhage, rupture,
Figure 7 Case 2- A Doppler Sonography demonstrating the
blood flow in the mass.
Figure 8 Case 2- The Ultrasound verifies the absence of
recurrence.
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infection and lymphorrhya. In the clinical examination
they are usually described as soft and co mpressible
masses, loculated in pattern. Histologically, hemo-lym-

phangiomas consist of dense fibrous tissue that grows in
bands between the numerous vascular spaces and
invades the subcutaneous fat. Some of those are blood
vessels and the others lymphatic.
Imaging modalities, Ultrasonography, Computed
Tomography and Magnetic Resonance, are useful in
confirming the diagnosis, describing the margins of the
mass and planning the surgical strategy[10]. The ultra-
sound, that is a low cost modality, may demonstrate the
solid or cystic nature of the lesion, therefore should be
ordered in a routine base; the extension and the rela-
tionship of the tumor to t he surrounding tissues is
rather depicted by the MRI [11]. The differential diagno-
sis includes hemangioma, lipoma, teratoma, congenital
lymphedema, dermoid cyst and neurofibroma. Needle
aspiration or biopsy can also be useful in the differen-
tiating hemo-lymphangioma to other fluid-filled masses.
As far as it concerns the therapeutic approach of loca-
lized hemo-lymphangiomas, the treatment of choice is
comp lete surgical excision, which also presents the low-
est recurrence rate. An important issue regards th e time
of surgery. Some authors express the aspect that the
mass should be excised promptly after being diagnosed,
while others prefer to wait and examine the size regu-
larly. In the latter case, the risk of infection and hemor-
rhage remains, while complete surgical eradication may
become more difficult. Other techniques available are:
aspirat ion and drain age, cryotherapy, injection of sclero-
tic agents and radiotherapy (by radium, roentgen ray or
radon seed), but none of them produced acceptable

results. Radiotherapy is used when surgical excision is
not feasible; the radio-sensitivity of hemo-lymphangio-
mas is not well understood, however in the past they
were considered to be radio-resistant. In children, radio-
therapy may lead to tumor retardation or to malignant
transformation [12]. When surgical eradication fails and
the mass recurs, conservative treatment methods may
be applied. The recurrence rates vary depending on the
complexity of the mass, the anatomical location and the
adequacy of the excision. However, lesions that have
been completely excised, present 10-27% recurrence,
while those being partially resected may recur in 50-
100%.
An important issue remains t he continuous lymphor-
rhya during the instant post-operative period; attentive
en bloc excision of the tumor, followed by rigorous cau-
terization of the blood and lymphatic vessels may reduce
or eliminate the risk of recurrence. Other auxiliary mea-
sures are the application of a bandage under pressure,
the prolonged drainage and immobilization of the
extremity.
Conclusions
In the treatment of hemo-lymphangioma, surgical exci-
sion appears to be the best choice of treatment, espe-
cially when the tumor increases in size, creating
pressure to the surrounding tissues. Eradicate attentive
excision offers the minimum risk of recurrence.
Consent
Written informed consent was obtained from the
patients for publication of this case report and any

accompanying images. A copy of the written consent is
available for review by the Editor-in-Chief of this journal.
Author details
1
Orthopaedic Clinic, Penteli’s Children Hospital, Athens, Greece.
2
Mitera”
General Maternity Hospital, Athens, Greece.
Authors’ contributions
IK, who is the corresponding author, was the surgeon of the first patient,
gathered the articles used as references and compiled the manuscript. M.V
was the surgeon of the second patient. A.K helped with the editing of the
manuscript. KF, as the director of the orthopaedic department, guided us
and helped in finalizing the manuscript.
All authors have read and approved the final manuscript
Competing interests
The authors declare that they have no competing interests.
Received: 29 June 2010 Accepted: 12 August 2010
Published: 12 August 2010
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doi:10.1186/1749-799X-5-56
Cite this article as: Kosmidis et al.: Hemolymphangioma of the lower
extremities in children: two case reports. Journal of Orthopaedic Surgery
and Research 2010 5:56.
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